Pulmonary circulation
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Pulmonary circulation · Jul 2020
Right ventriculo-pulmonary arterial uncoupling and poor outcomes in pulmonary arterial hypertension.
Right ventricular function critically affects the prognosis of patients with pulmonary arterial hypertension. We aimed to analyze the prognostic value of right ventricular indices calculated using magnetic resonance imaging and right heart catheterization metrics in pulmonary arterial hypertension. We retrospectively collected data from 57 Japanese patients with pulmonary arterial hypertension and 18 controls and calculated six indices of right ventricular function: two indices of contractility (end-systolic elastance calculated with right ventricular maximum pressure and with magnetic resonance imaging metrics); two indices of right ventricular-pulmonary arterial coupling (end-systolic elastance/arterial elastance calculated with the pressure method (end-systolic elastance/arterial elastance (P)) and with the volume method (end-systolic elastance/arterial elastance (V)); and two indices of right ventricular diastolic function (stiffness (β) and end-diastolic elastance). ⋯ According to the multivariate Cox regression analysis, decreased end-systolic elastance/arterial elastance (P) was associated with a higher composite event rate (hazard ratio 11.510, 95% confidence interval: 1.954-67.808). In conclusion, an increased right ventricular contractility, diastolic dysfunction, and a trend of impaired right ventricular-pulmonary arterial coupling were observed in our pulmonary arterial hypertension cohort. According to the multivariate outcome analysis, a decreased end-systolic elastance/arterial elastance (P), suggestive of impaired right ventricular-pulmonary arterial coupling, best predicted the pulmonary arterial hypertension-related event.
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Pulmonary circulation · Jul 2020
Characteristics of Japanese elderly patients with pulmonary arterial hypertension.
Previous nationwide Japanese data suggested that pulmonary arterial hypertension (PAH) predominantly affects young women. However, the number of elderly patients diagnosed with PAH has been increasing in western countries. There have been no reports on elderly PAH patients in Asian countries. ⋯ Elderly patients in Study 2 showed less improvement in hemodynamics with therapy. There was no significant difference in disease-specific survival between elderly and younger patients. Japanese elderly patients with I/H-PAH showed poorer exercise capacity and impaired gas exchange, but better pulmonary hemodynamics than younger patients.
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Pulmonary circulation · Jul 2020
Current clinical utilization of risk assessment tools in pulmonary arterial hypertension: a descriptive survey of facilitation strategies, patterns, and barriers to use in the United States.
Practice guidelines suggest that treatment decisions in pulmonary arterial hypertension be informed by periodic assessment of patients' clinical risk. Several tools, well validated for risk discrimination, such as the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management calculator, were developed to assess pulmonary arterial hypertension patients' risk of death based on multiple parameters, including functional class, hemodynamics, biomarkers, comorbidities, and exercise capacity. Using an online survey, we investigated the use of risk assessment tools by pulmonary hypertension healthcare providers in the United States. ⋯ Risk was most frequently assessed by decision makers at the time of diagnosis (cited by 54%) and at the time of worsening symptoms (cited by 42%), suggesting that use of pulmonary arterial hypertension risk assessment tools remains low. In our survey, non-physicians compared with physicians cited two major barriers to increased tool use: lack of education and training (20% vs. 4%) and lack of clarity on the best tool to use (30% vs. 18%). Information technology tools, such as electronic medical record integration and web or phone-based risk calculating applications, were cited most frequently as ways to increase the use of risk assessment tools.
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Pulmonary circulation · Jul 2020
Case ReportsA novel BMPR2 mutation with widely disparate heritable pulmonary arterial hypertension clinical phenotype.
Mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2) have been associated with heritable pulmonary arterial hypertension (HPAH), whereas mutations in the gene encoding eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) are associated with heritable pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (HPVOD/PCH). We describe two unrelated patients found to carry the same hitherto unreported pathogenic BMPR2 mutation; one of whom presented with typical pulmonary arterial hypertension, whereas the second patient presented with aggressive disease and characteristic clinical features of PVOD/PCH. These two clinically divergent cases representative of the same novel pathogenic mutation exemplify the variable phenotype of HPAH and the variable involvement of venules and capillaries in the pathology of the pulmonary vascular bed in pulmonary arterial hypertension.
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Pulmonary circulation · Jul 2020
Relationship of left ventricular outflow tract velocity time integral to treatment strategy in submassive and massive pulmonary embolism.
Pulmonary embolism is associated with high rates of mortality and morbidity. It is important to understand direct comparisons of current interventions to differentiate favorable outcomes and complications. The objective of this study was to compare ultrasound-accelerated thrombolysis versus systemic thrombolysis versus anticoagulation alone and their effect on left ventricular outflow tract velocity time integral. ⋯ In this retrospective study of submassive or massive pulmonary embolisms, left ventricular outflow tract velocity time integral demonstrated greater improvement in patients treated with ultrasound-accelerated thrombolysis as compared to anticoagulation alone, a finding not seen with systemic thrombolysis. While this improvement in left ventricular outflow tract velocity time integral parallels the trend seen in mortality outcomes across the three groups, it only correlates with changes seen in pulmonary artery systolic pressure, not in other markers of echocardiographic right ventricular dysfunction (tricuspid annular plane systolic excursion and right ventricular to left ventricular ratios). Changes in left ventricular outflow tract velocity time integral, rather than echocardiographic markers of right ventricular dysfunction, may be considered a more useful prognostic marker of both dysfunction and improvement after reperfusion therapy.